PDF(Pubmed)
Abstract：
Anti-melanoma differentiation-associated protein 5 antibody-positive dermatomyositis (anti-MDA5-DM) is frequently complicated by progressive interstitial lung disease (ILD), the prognosis of which is poor, and management is a major challenge. We treated three patients with anti-MDA5-DM-associated ILD (anti-MDA5-DM-ILD) using the Janus kinase (JAK) inhibitor, baricitinib, which improved lung opacities and saved two patients. We reviewed 6 patients with anti-MDA5-DM-ILD who had been treated with tofacitinib at our institution. Five of the patients survived, although discontinuation of tofacitinib due to complications was frequently observed. In addition, a literature search of patients with anti-MDA5-DM-ILD who were treated with JAK inhibitors yielded 21 articles involving 79 cases. All patients except one were treated with tofacitinib, and the survival rate was 75.9%. Although not statistically confirmed, the deceased patients tended to be older and had higher ferritin levels. A total of 92 complications were observed, 11 of which resulted in JAK inhibitor discontinuation. Cytomegalovirus reactivation comprised a substantial percentage of all complications and of those patients who required JAK inhibitor discontinuation. Five cases with fatal infective complications were also observed. While tofacitinib has been proposed to be a therapeutic option for anti-MDA5-DM-ILD, other JAK inhibitors, including baricitinib, are a treatment option. Further investigation is warranted to optimize treatment of anti-MDA5-DM-ILD.
摘要：
抗黑素瘤分化相关蛋白5抗体阳性皮肌炎（抗MDA5-DM）经常并发进行性间质性肺病（ILD），其预后较差，管理是一个重大挑战。我们使用Janus激酶（JAK）抑制剂治疗了3例抗MDA5-DM相关ILD（抗MDA5-DM-ILD）患者，baricitinib,改善了肺部混浊并挽救了两名患者。我们回顾了在我们机构接受托法替尼治疗的6例抗MDA5-DM-ILD患者。五名患者幸存下来，尽管经常观察到由于并发症而停用托法替尼。此外,通过对接受JAK抑制剂治疗的抗MDA5-DM-ILD患者的文献检索,获得了21篇文献,共79例.除一名患者外，所有患者均接受了托法替尼治疗，生存率为75.9%。虽然没有统计证实，死亡患者往往年龄较大，铁蛋白水平较高。共观察到92例并发症，其中11例导致JAK抑制剂停用。巨细胞病毒再激活占所有并发症和需要JAK抑制剂停药的患者的相当大百分比。还观察到5例致命的感染并发症。虽然托法替尼已被提议作为抗MDA5-DM-ILD的治疗选择，其他JAK抑制剂，包括Baricitinib,是一种治疗选择。需要进一步研究以优化抗MDA5-DM-ILD的治疗。
